Autoimmune Haemolyitic Anaemia

ORPHANET code: 98375
ICD-10 code: D59.0 D59.1
Group: Acquired hemolytic anemia
- What causes the disease and how common is it?
Autoimmune hemolytic anèmia (AIHA) or immunohaemolytic anaemia (IHA) occurs when antibodies directed against the person’s own red blood cells (RBCs) cause them to burst (lyse), leading to anaemia of variable severity. This happens because the immune system mistakenly recognizes these blood cells as foreign.The intracellular components of the RBCs are released into the circulating blood and into tissues, leading to some of the characteristic symptoms of this condition. Possible causes include: Certain chemicals, drugs, and toxins, Infections, transfusion of blood from a donor with a blood type that does not match, and certain càncers. When antibodies form against red blood cells for no reason, the condition is called idiopathic AIHA. AIHA is a relatively rare condition, affecting one to three people per 100,000 per year. - What are the most frequent symptoms if I have the disease?
If anaemia is mild patient may not have symptoms. If the AIHA develops slowly, symptoms that may occur and include: Feeling grumpy, weak or tired more often than usual, especialy after slight exercise, headaches and difficult for concentrating or thinking. If the anemia gets worse, symptoms may include , brittle nails, light-headedness when you stand up, Pale skin color (pallor), shortness of breath and sore tonge. - Which treatment must I follow if I have the disease?
The first treatment tried is usually a steroid medication, such as prednisone. If steroid medications do not improve the condition, treatment with intravenous immunoglobulin (IVIG) or removal of the spleen (splenectomy) may be considered. if you do not respond to steroids and splenectomy. treatment to suppress immune System is required. Medications such as azathioprine (Imuran), cyclophosphamide (Cytoxan), and rituximab (Rituxan) have been used. Blood transfusions are given whe necessary but they have to be given with caution, because the blood may not be compatible and it may cause more red blood cell destruction. - What is the risk of passing the condition on to my children?
This is an acquired disorder.
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